Korean J Dermatol.  2016 Jun;54(5):377-380.

Congenital Dermatofibrosarcoma Protuberans Treated with Wide Excision

• Park SK
• Woo SH
• Kim DW
• Hwang SR
• Park J
• Yun SK
• Kim HU

Abstract

Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor with intermediate malignancy that usually appears in adults. The congenital type is very rare, and only three cases have been reported so far in the Korean literature. A 2-month-old girl presented with a firm reddish-gray nodule on the middle of her back that had been present since birth. Histopathologically, the lesion was composed of fascicles of densely packed uniform spindle cells arranged in a storiform (mat-like) pattern. The cells had hyperchromatic nuclei and rare mitotic figures. On immunohisto-chemistry, the tumor cells were positive for CD34. Based on these findings, we made the diagnosis of congenital dermatofibrosarcoma protuberans. She underwent wide excision, and there was no evidence of recurrence during 24 months of follow-up. Our report is of interest because of the rarity of congenital dermatofibrosarcoma protuberans and its earliest detection reported in Korea.

Keyword

Congenital dermatofibrosarcoma protuberans; Wide excision

MeSH Terms

Adult
Dermatofibrosarcoma*
Diagnosis
Female
Follow-Up Studies
Humans
Infant
Korea
Parturition
Recurrence